Quality of life and functional capacity can be improved in patients with Eisenmenger syndrome with oral sildenafil therapy.

BACKGROUND: Patients with Eisenmenger syndrome (ES) have a decreased exercise capacity and poor quality of life (QoL). While patients may survive to middle adulthood, the burden of disease is disabling. Sildenafil seems to improve exercise tolerance and hemodynamics, but there is no data to date on its impact on QoL. METHODS: Eisenmenger patients in New York Heart Association (NYHA) class III were recruited in a prospective study of efficacy and safety of oral sildenafil. The QoL endpoint was assessed using a disease-specific questionnaire (CAMPHOR). Exercise capacity was assessed by means of six minute walk test (6MWT). All patients underwent comprehensive assessment at baseline and after 3months of treatment. RESULTS: Twelve patients (mean age was 34.3±10.2, 83% female) with various cardiac anatomies were recruited. No major adverse events during the follow-up or significant drop in resting oxygen saturation were recorded. After 3months of oral sildenafil therapy, all patients improved to NYHA II with a concomitant improvement in 6MWT distance (347.3±80.7 to 392.5±82.0m, p=0.002). All components of the CAMPHOR score, relating to symptoms, activity and QoL, improved significantly resulting in substantial improvement in the total CAMPHOR score (27.6±10.5 to 15.8±10.4, p=0.002). CONCLUSIONS: Three months of sildenafil therapy in adults with ES was well tolerated and associated with significant improvement in the QoL CAMPHOR questionnaire and in NYHA class and exercise capacity. Larger studies are warranted to assess long term efficacy of oral sildenafil and potential impact on survival.

Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome.

INTRODUCTION: Iron deficiency is common in cyanotic congenital heart disease (CHD) and results in reduced exercise tolerance. Currently, iron replacement is advocated with limited evidence in cyanotic CHD. We investigated the safety and efficacy of iron replacement therapy in this population. METHODS: Twenty-five iron-deficient cyanotic CHD patients were prospectively studied between August 2008 and January 2009. Oral ferrous fumarate was titrated to a maximum dose of 200mg thrice-daily. The CAMPHOR QoL questionnaire, 6 minute walk test (6MWT) and cardiopulmonary exercise testing were conducted at baseline and after 3 months of treatment. RESULTS: Mean age was 39.9 ± 10.9 years, 80% females. Fourteen had Eisenmenger syndrome, 6 complex cyanotic disease and 5 Fontan circulation. There were no adverse effects necessitating termination of treatment. After 3 months of treatment, hemoglobin (19.0 ± 2.9 g/dL to 20.4 ± 2.7 g/dL, p<0.001), ferritin (13.3 ± 4.7 µg/L to 54.1 ± 24.2 µg/L, p<0.001) and transferrin saturation (17.8 ± 9.6% to 34.8 ± 23.4%, p<0.001) significantly increased. Significant improvements were also detected in the total CAMPHOR score (20.7 ± 10.9 to 16.2 ± 10.4, p=0.001) and 6MWT distance (371.7 ± 84.7 m to 402.8.0±74.9m, p=0.001). Peak VO(2) remained unchanged (40.7 ± 9.2% to 43.8 ± 12.4% of predicted, p=0.15). CONCLUSION: Three months of iron replacement therapy in iron-deficient cyanotic CHD patients was safe and resulted in significant improvement in exercise tolerance and quality of life. Identification of iron deficiency and appropriate replacement should be advocated in these patients.

A rare type of double aortic arch diagnosed by echocardiography in an asymptomatic 9-year-old patient.

A case of double aortic arch with an atretic left arch distal to the origin of the left subclavian artery was suspected by echocardiography (ECHO) in an asymptomatic 9-year-old girl and confirmed by magnetic resonance imaging (MRI). The authors report their ECHO findings, which could be an important diagnostic tool for symptomatic patients because confusion exists in the literature regarding the differentiation of the aforementioned rare malformation from a right arch with mirror image branching. The authors support their ECHO proposal with an explanation based on the hypothetical double aortic arch plan set by Edwards.

Burden of coronary artery disease in adults with congenital heart disease and its relation to congenital and traditional heart risk factors.

As adult patients with congenital heart disease (CHD) grow older, the risk of developing coronary artery disease (CAD) increases. We sought to estimate the prevalence of CAD in adult patients with CHD, the safety of coronary angiography in this setting, and the potential relation of CAD to clinical and hemodynamic parameters. Two hundred fifty adult patients with CHD (mean age 51 +/- 15 years; 53% men) underwent selective coronary angiography in our center for reasons other than suspected CAD. Clinical and hemodynamic data were retrieved retrospectively from medical records and echocardiographic and angiographic databases, respectively. Significant CAD using quantitative coronary angiography was found in 9.2% of adult patients with CHD. No patient with cyanosis or age <40 years had significant CAD. Systolic and diastolic systemic ventricular dimensions were significantly higher in patients with CAD, even after adjustment for age (odds ratio [OR] for 10-mm increase 2.59, 95% confidence interval [CI] 1.29 to 5.21, p = 0.007; OR 2.31, 95% CI 1.24 to 4.31, p = 0.008, respectively). Systemic arterial hypertension and hyperlipidemia were strong predictors of CAD (OR 4.54, 95% CI 1.82 to 12.0, p = 0.001; OR 9.08, 95% CI 3.56 to 24.54, p <0.0001, respectively), whereas no relation to chest pain was found. Only 1 major adverse event was recorded during coronary angiography. In conclusion, the prevalence of significant CAD in a hospital adult CHD cohort was similar to that in the general population. This study supported the performance of selective coronary angiography in patients >40 years referred for cardiac surgery, with low risk of major complications. Traditional cardiovascular risk factors for CAD also applied to adult patients with CHD, in whom primary prevention of CAD was as important as in the general population.